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Von Willebrand disease

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https://www.readbyqxmd.com/read/28822718/role-of-the-coagulation-system-in-genitourinary-cancers-review
#1
REVIEW
Axel John, Christian Gorzelanny, Alexander T Bauer, Stefan W Schneider, Christian Bolenz
Tumor progression is associated with aberrant hemostasis, and patients with malignant diseases have an elevated risk of developing thrombosis. A crosstalk among the vascular endothelium, components of the coagulation cascade, and cancer cells transforms the intravascular milieu to a prothrombotic, proinflammatory, and cell-adhesive state. We review the existing evidence on activation of the coagulation system and its implication in genitourinary malignancies and discuss the potential therapeutic benefit of antithrombotic agents...
July 26, 2017: Clinical Genitourinary Cancer
https://www.readbyqxmd.com/read/28820407/-evaluation-of-parameters-of-hemostasis-in-the-third-trimester-in-pregnant-women-with-congen%C3%A4-tal-hemorrhagic-diathesis
#2
L Rzaguliyeva, G Afandiyeva, N Malikqasımova
The aim is to study the coagulogram indices in the III trimester of pregnancy in women with hemophilia and von Willebrand disease. Twenty-seven pregnant women with hereditary coagulopathies of mild and moderate form (mean age 27.9±3.3 years) in the third trimester of pregnancy were examined. Of the examined 12 women - carriers of hemophilia A and B, 15 - with Willebrand disease (BV). Clinico-laboratory examination, clinical and biochemical blood tests were carried out. Coagulologic examination was performed on 17 parameters...
July 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28806915/evaluating-the-maintenance-of-disease-associated-variation-at-the-blood-group-related-gene-b4galnt2-in-house-mice
#3
Marie Vallier, Maria Abou Chakra, Laura Hindersin, Miriam Linnenbrink, Arne Traulsen, John F Baines
BACKGROUND: B4galnt2 is a blood group-related glycosyltransferase that displays cis-regulatory variation for its tissue-specific expression patterns in house mice. The wild type allele, found e.g. in the C57BL/6 J strain, directs intestinal expression of B4galnt2, which is the pattern observed among vertebrates, including humans. An alternative allele class found in the RIIIS/J strain and other mice instead drives expression in blood vessels, which leads to a phenotype similar to type 1 von Willebrand disease (VWD), a common human bleeding disorder...
August 14, 2017: BMC Evolutionary Biology
https://www.readbyqxmd.com/read/28806860/patient-caregiver-and-provider-perceptions-of-pain-and-pain-management-in-adolescents-and-young-adults-with-bleeding-disorders
#4
Angela Lambing, Cynthia D Nichols, James E Munn, Terry L Anderson, Bartholomew J Tortella, Michelle L Witkop
INTRODUCTION: Recurrent bleeding and associated pain are critical components in the management of bleeding disorders, yet scant data describe perceptions of pain in this patient population. OBJECTIVE: This study assessed perceptions of pain and pain management in adolescents and young adults (AYAs) with haemophilia or von Willebrand disease (VWD) to determine agreement/disagreement between patients, caregivers and health care providers. METHODS: Using an online questionnaire, AYA patients (N=89), their caregivers (N=77), and providers (N=54) reported on pain perception, pain treatment and pain control...
August 14, 2017: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/28804850/laboratory-testing-for-von-willebrand-factor-multimers
#5
Susan Oliver, Kun Kan Edwin Lau, Kent Chapman, Emmanuel J Favaloro
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. As an additional step, an evaluation of VWF structural features by multimer analysis is useful in selective investigations...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804849/ristocetin-induced-platelet-aggregation-ripa-and-ripa-mixing-studies
#6
Juan Pablo Frontroth, Emmanuel J Favaloro
Ristocetin-induced platelet aggregation (RIPA) is used as an in vitro test to determine the presence and integrity of the platelet glycoprotein (GP) Ibα-V-IX complex and von Willebrand factor (VWF) interaction and is usually performed using platelet-rich plasma (PRP). Impairment in the response of VWF/GPIbα-V-IX is measured with reference to several established concentrations of ristocetin and may indicate defects in VWF or in GPIbα-V-IX function. RIPA-based mixing studies comprise an additional approach to testing this interaction to help define whether defects identified by RIPA lie in VWF or in GPIbα-V-IX...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804848/laboratory-testing-for-von-willebrand-factor-factor-viii-binding-for-2n-vwd
#7
Soma Mohammed, Emmanuel J Favaloro
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes a protocol for assessment of VWF activity by means of VWF: factor VIII binding (VWF:FVIIIB)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804847/laboratory-testing-for-von-willebrand-factor-activity-by-glycoprotein-ib-binding-assays-vwf-gpib
#8
Jürgen Patzke, Emmanuel J Favaloro
In addition to assessment of von Willebrand factor (VWF) antigen (VWF:Ag), the first-line laboratory investigation of possible von Willebrand disease (VWD) often includes an assay to measure GPIb (glycoprotein Ib) binding activity of VWF. A decreased GPIb binding activity is characteristic for most of the VWD types. For many years, the most frequently used assay for measuring GPIb binding activity was the ristocetin cofactor assay (VWF:RCo), which measures the agglutination of fixed human platelets by VWF in the presence of ristocetin...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804846/laboratory-testing-for-von-willebrand-factor-ristocetin-cofactor-vwf-rco
#9
Soma Mohammed, Emmanuel J Favaloro
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for these VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes several protocols for assessment of VWF activity by means of VWF ristocetin cofactor (VWF:RCo)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804845/laboratory-testing-for-von-willebrand-factor-collagen-binding-vwf-cb
#10
Emmanuel J Favaloro, Soma Mohammed
Von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders develop due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. This chapter describes several protocols for assessment of VWF activity by means of VWF collagen binding (VWF:CB)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804844/laboratory-testing-for-von-willebrand-factor-antigen-vwf-ag
#11
Emmanuel J Favaloro, Soma Mohammed, Jürgen Patzke
von Willebrand disease (VWD) is reportedly the most common inherited bleeding disorder and can also arise as an acquired syndrome (AVWS). These disorders arise due to defects and/or deficiency of the plasma protein von Willebrand factor (VWF). Laboratory testing for the VWF-related disorders requires assessment of both VWF level and VWF activity, the latter requiring multiple assays because of the many functions carried out by VWF to help prevent bleeding. The current paper describes protocols for assessment of VWF level by means of VWF antigen (VWF:Ag)...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28804843/diagnosis-or-exclusion-of-von-willebrand-disease-using-laboratory-testing
#12
Emmanuel J Favaloro
von Willebrand disease (VWD) is a common bleeding disorder diagnosed based on clinical features and following laboratory testing. VWD is due to deficiencies or defects in the plasma protein von Willebrand factor (VWF), a large adhesive protein with multiple activities. Laboratory testing therefore centers on assessment of VWF protein level using VWF antigen (VWF:Ag), as well as assays that measure VWF activity, most notably platelet glycoprotein (GP) Ib and collagen binding (VWF:CB) activities. Decreases in VWF:Ag and VWF activities, as well as the pattern of such changes, help define VWD and its type...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28802375/post-tonsillectomy-hemorrhagic-outcomes-in-children-with-bleeding-disorders-at-a-single-institution
#13
Priyesh N Patel, Alexandra M Arambula, Allison P Wheeler, Edward B Penn
OBJECTIVE: To report on the post-tonsillectomy bleeding outcomes and factors associated with hemorrhage among children with pre- or post-operatively diagnosed bleeding disorders treated with an institutional protocol. METHODS: Retrospective cohort study of patients with hematologic disorders who underwent tonsillectomy between 2003 and 2016 and were treated with perioperative desmopressin or factor replacement and/or aminocaproic acid. Postoperative outcomes were compared to controls matched for age, sex, and indication for surgery...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28791655/current-and-emerging-options-for-the-management-of-inherited-von-willebrand-disease
#14
REVIEW
Jessica M Heijdra, Marjon H Cnossen, Frank W G Leebeek
Von Willebrand disease (VWD) is the most common inherited bleeding disorder with an estimated prevalence of ~1% and clinically relevant bleeding symptoms in approximately 1:10,000 individuals. VWD is caused by a deficiency and/or defect of von Willebrand factor (VWF). The most common symptoms are mucocutaneous bleeding, hematomas, and bleeding after trauma or surgery. For decades, treatment to prevent or treat bleeding has consisted of desmopressin in milder cases and of replacement therapy with plasma-derived concentrates containing VWF and Factor VIII (FVIII) in more severe cases...
August 8, 2017: Drugs
https://www.readbyqxmd.com/read/28789675/life-threatening-subdural-hematoma-after-aortic-valve-replacement-in-a-patient-with-heyde-syndrome-a-case-report
#15
Tetsuro Uchida, Azumi Hamasaki, Eiichi Ohba, Atsushi Yamashita, Jun Hayashi, Mitsuaki Sadahiro
BACKGROUND: Heyde syndrome is known as a triad of calcific aortic stenosis, anemia due to gastrointestinal bleeding from angiodysplasia, and acquired type 2A von Willebrand disease. This acquired hemorrhagic disorder is characterized by the loss of the large von Willebrand factor multimers due to the shear stress across the diseased aortic valve. The most frequently observed type of bleeding in these patients is mucosal or skin bleeding, such as epistaxis, followed by gastrointestinal bleeding...
August 8, 2017: Journal of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/28775254/adamts-13-regulates-neutrophil-recruitment-in-a-mouse-model-of-invasive-pulmonary-aspergillosis
#16
Astrid Alflen, Steve Prüfer, Katharina Ebner, Sebastian Reuter, Pamela Aranda Lopez, Inge Scharrer, Fumiaki Banno, Michael Stassen, Hansjörg Schild, Kerstin Jurk, Markus Bosmann, Hendrik Beckert, Markus P Radsak
Von Willebrand factor (VWF) is secreted as an acute phase protein during inflammation. ADAMTS-13 regulates the size and prothrombotic activity of VWF by it's specific proteolytic activity. To determine the relevance of this regulatory pathway for the innate inflammatory response by polymorphonuclear neutrophils (PMN), we employed a mouse model of invasive pulmonary aspergillosis (IPA) where PMN functionality is crucial for fungal clearance and survival. IPA was induced by intratracheal application of Aspergillus fumigatus (A...
August 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28775074/stasis-promotes-erythrocyte-adhesion-to-von-willebrand-factor
#17
Michel W J Smeets, Marjon J Mourik, Hans W M Niessen, Peter L Hordijk
OBJECTIVE: Venous thromboembolism is a major contributor to global disease burden. Leukocytes and platelets initiate thrombogenesis on blood stasis and initiate the formation of a fibrin, VWF (von Willebrand factor), and neutrophil extracellular trap scaffolds for erythrocytes. However, there is little knowledge on how erythrocytes become stably incorporated into this scaffold. Recently, we described the adhesion of calcium-loaded erythrocytes to endothelial-derived VWF strings. Because VWF is part of the scaffold of venous thrombi, we questioned whether reduced flow or stasis promotes the adhesion of normal erythrocytes to VWF and whether venous thrombi show evidence of erythrocyte-VWF interactions...
August 3, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28774197/thrombotic-and-hemorrhagic-conditions-due-to-a-gain-of-function-of-coagulation-proteins-a-special-type-of-clotting-disorders
#18
Antonio Girolami, Elisabetta Cosi, Silvia Ferrari, Annamaria Lombardi, Fabrizio Fabris
Coagulation disorders can be classified into 2 types, namely, type I and type II. In the former, there is a concomitant decrease in factor activity and antigen (activity-antigen ratio is 1), whereas in the latter, there is a discrepancy between factor activity which is always low and antigen which is normal or near normal (activity-antigen ratio is <1, eg, 0.5). Recently, several gain-of-function disorders have been described. These are characterized by an increased activity with respect to the antigen level...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28765701/levels-and-activities-of-von-willebrand-factor-and-metalloproteinase-with-thrombospondin-type-1-motif-number-13-in-inflammatory-bowel-diseases
#19
Dorota Cibor, Danuta Owczarek, Saulius Butenas, Kinga Salapa, Tomasz Mach, Anetta Undas
AIM: To evaluate the levels of von Willebrand factor (VWF) and metalloproteinase with thrombospondin type-1 motif, number 13 (ADAMTS13) in inflammatory bowel disease (IBD) and correlate them with the disease activity. METHODS: Consecutive patients with IBD aged 18 years or older were enrolled in the study. Forty-seven patients with ulcerative colitis (UC), 38 with Crohn's disease (CD), and 50 healthy controls were included. The white blood cell count, haematocrit, platelet count, fibrinogen, partial activated thromboplastin time, C-reactive protein, albumin, VWF antigen level (VWF:Ag), VWF ristocetin cofactor activity (VWF:RCo), VWF collagen-binding activity (VWF:CB), and ADAMTS13 antigen level (ADAMTS13:Ag) and activity (ADAMTS13act) were measured...
July 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28759961/lumbar-sympathectomy-regulates-vascular-cell-turnover-in-rat-hindfoot-plantar-skin
#20
Zhifang Zheng, Yu Wan, Yishu Liu, Lulu Zhu, Jianbing Tang, Wenhua Huang, Biao Cheng
BACKGROUND: Sympathetic denervation and impaired angiogenesis cause skin diseases. However, the relationship between the sympathetic nervous system and vascular cell turnover in normal skin remains unclear. OBJECTIVE: To determine the effects of sympathetic denervation on vascular cell turnover in normal skin. METHODS: Rats underwent bilateral L2-4 sympathetic trunk resection (sympathectomy group) or sham operation (control). Hindfoot plantar skin was analyzed 2 weeks and 3 months postoperatively...
July 25, 2017: Clinical Hemorheology and Microcirculation
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